Autoimmune Pancreatitis
童綜合醫院 劉忠政醫師
Admission Summary
• A 40-year-old women is an aphasia . • This time , jaundice and itching sensation for about 20 days were noted . • At first she was brought to 竹山秀傳醫院 • The abdominal echo and CT showed severe pancreatic swelling and R/O tumor • She was transferred to our hospital for help .
Physical Examination
• Conscious clear , BP : 99/66 mmHg PR : 60 . BT : 36.9 , BW : 60 Kg , height:148cm • Conjunctiva : no pale , sclera : severe icteric • Abdomen : soft and flat , no palpable mass liver/spleen impalpable • no pitting edema • Skin : yellowish discoloration
Laboratory Data
• WBC : 8600 Hgb : 11.0 PLT : 341000 • Sugar : 198 , r-GT : 305 , AlK-P : 244 GOT/GPT :44/31 Bilirubin T/D : 19.0/15.5 • Amylase : 41 , lipase : 21 • BUN/Cr : 7.2/0.6 Na/K : 132/3.3 • HbsAg (-) , anti-HCV (-) • AFP , CEA , CA-199 : WNL • IgG : 2820 ( 650-1600 ) , ANA : 1: 160 x (+) • ASMA (-) , RA factor (-)
2007-1-16 sono
EUS
• 1.Diffusely enlarged pancreas , mainly hypoechoic pattern , hyperechoic spots were seen inside pancreas parenchyma • 2.Beaded appearance of MPD(+) • 3.Peripancreas LN , 1.4 cm , hypoechoic • 4.CBD : 1.8 , 5.Postbulb ulcer (+) • Autoimmune pancreatitis with CBD obstruction is most favored
2007-1-16 ERCP
2007-1-16 ERCP
Differential diagnosis
• Obstructive jaundice : Alcohol history (-) HbsAg (-) , anti-HCV (-), stone (-) tumor(-) • Image study : Abdominal CT : Diffusely enlarged pancrease ERCP : diffuse pancreatic ductal narrowing • IgG : 2820 ( 650-1600 ) ,ANA : 1: 160 x (+) • Autoimmune pancreatitis is impression
Treatment
• PTCD for drainage • High dose corticosteroid ( prednisolon ) for 8 weeks and slowly taper • ( Prednisolon 2 # Tid x 14 days (1/19-2/1) 2# Bid x 14 days ( 2/2 -2/15 ) 1#Tidx 7 days ( 2/16-2/22 ) 1# Bid x 7 days (2/23-3/1) 1# QD ( 3/2 maintain dose )
Clinical Course
• After PTCD and steroid therapy , jaundice much better • Bilirubin ( 96/1/15 ) 19.0 (1/17) 15.1 (1/19) 8.8(1/26) 6.3 (2/2) 4.0(2/7) 2.6(3/9)1.2 (3/23) 1.0 (4/13) 2.1(5/25) 2.0 • IgG : (96/1/17) 2820 (96/2/7) 1490 ANA : 1: 160 x (+) 1:40 (-) • Abdominal echo also show improvement
3.08cm
1.39cm
2007-2-5 sono
2.37cm
Autoimmune Pancreatitis (AIT )
• Was described by Sarles et al over 40 years ago . • Was introduced by Yoshida et al in 1995 . • Chronic pancreatitis that is associated with autoimmune manifestations revealed on laboratory , histologic and clinical testing. • Treatment with corticosteroids leads to the rapid and sustained symptoms .
Epidemiology
• Autoimmune pancreatitis is rare . • Three series have reported the prevalence of autoimmune pancreatitis as between 5% and 6 % of all patients with chronic pancreatitis • Men : women = 2:1 • Most are older than 50 years
Cause and Pathogensis
• The cause of autoimmune pancreatitis is unknown • Frequently associated with rheumatoid arthritis , Sjogren’s syndrome , inflammatory bowel disease
Clinical Features
• In a large series , 63 % of the patients had jaundice and 35 % of patients had abdominal pain . • Most symptoms associated with autoimmune pancreatitis are responsive to corticosteroid therapy
Diagnostic Criteria
• Proposed by Japan Pancreas Society • Combination of imaging , laboratory testing and histologic analysis .
Diagnostic Imaging
• Abdominal CT : diffuse pancreatic enlargement , some case had low-attenuation mass in the head of pancreas • ERCP ( or MRCP ) : Focal , diffuse or segmental narrowing of the main pancreatic duct
NEJM
Figure 2. CT Scan Showing Typical Features of Autoimmune Pancreatitis: Diffuse Enlargement of the Pancreas with Homogeneous Attenuation and the Peripheral Rim of a Hypoattenuation "Halo" (Arrow).
Figure 3. Findings on Endoscopic Retrograde Cholangiopancreatography before (Panel A) and after (Panel B) Prednisone Therapy
NEJM
Serologic analysis
• Elevated serum IgG4 level • Elevated serum IgG or gamma globumin level • Presence of ALA (anti-lactoferrin antibody) ANA , ASMA , ACA ( anti-carbonic anhydrase II antibody )
Pancreatic-biliary histologic analysis
• Periductal lympho-plasmacytic infiltration or fibrosis • Obliterative phlebitis • IgG4-postive plasma cells in tissue
Diagnosis criteria
• Abdominal CT : diffusely enlarged pancreas and ERCP : segmental pancreatic –ductal narrowing must considered of antoimmune pancreatitis • Increase IgG levels or autoantibodies is supportive of the diagnosis • Increase IgG4 level is nearly diagnosis
Biopsy
• Autoimmune pancreatitis can produce a mass in the head of the pancreas . • Can minic pancreatic adenocarcinoma . • Fine-needle aspiration of the mass may be helpful . • Corticosteroid therapy can be used as a diagnostic tool ( 2 to 4 weeks ) • Surgical biopsy of the mass if therapy fails
Treatment
• Prednisone 40 mg daily for 1 week , followed by a taper of 5mg per week . Maintain dose : prednisolon 5 to 10 mg per day . • The response of corticosteroids is often dramatic ( resolution of pancreatic mass and swelling , stricture and jaundice ) • IgG4 , hypergamaglobulinemia , and presence of autoantibodies may show improvement after 2-4 weeks therapy
Summary
• Autoimmue pancreatitis is an immunebased systemic disease • Diagnosis on the basis of imaging , serologic and histologic criteria • Is good responsive to corticosteroid therapy • Some case can mimic pancreatic head cancer , pancreatic biopsy will aid in the diagnosis.
Thanks for your attention
童綜合醫院 劉忠政醫師